Xanthomatosis

Osteoarticular lesion in xanthoma disseminatum treated with total hip arthroplasty: a case report

“…Xanthoma disseminatum is a rare form of normolipidemic, mucocutaneous xanthoma unaccompanied by hyperlipidemia; reactive granulomatous proliferative disorder of reticuloendothelial histiocytes is considered the cause [3, 4]. The condition was first reported in 1938 by Montgomery and Osterberg, and approximately 100 cases have been reported to date [5]. The age of onset varies widely from 1 to 70 years, but two-thirds or three-quarters of patients develop the disease before the age of 25 years.…”

Histiocytosis X

“…Such involvement may cause considerable distortion of the upper respiratory tract and this has occasionally necessitated tracheostomy or surgical removal. Xanthoma disseminata is usually associated with skin lesions and it should be noted that diabetes insipidus may also be present (Turner et al, 1925;Finney et al, 1932;New, 1935;Montgomery and Osterburg, 1938). Granulomatous changes in the larynx causing deep ulceration of the mucosa have also been described (Miiller, 1938).…”

Lipidproteinosis (Urbach-Wiethe Syndrome)

“…A disease which may cause difficulty in differential diagnosis is disseminated xanthomatosis (Montgomery and Osterberg, 1938). This usually begins in adult life and affects the upper respiratory mucosae and skin; papules, at first reddish but later yellowish-brown, may appear at the lid edges (Spiegel, 1938), but especially in the flexural creases and axillae; conjunctival infiltration and diabetes insipidus can also occur.…”

“…This usually begins in adult life and affects the upper respiratory mucosae and skin; papules, at first reddish but later yellowish-brown, may appear at the lid edges (Spiegel, 1938), but especially in the flexural creases and axillae; conjunctival infiltration and diabetes insipidus can also occur. On the other hand, lipidproteinosis begins in infancy, prefers the extensor surfaces of joints, does not involve the conjunctiva, and is occasionally accompanied by diabetes mellitus, but never by diabetes insipidus.…”